摘要
目的分析儿童肾移植受者移植后淋巴组织增生性疾病(PTLD)的临床特点及诊疗情况。方法回顾性分析2011年1月至2024年6月复旦大学附属儿科医院肾移植后并发PTLD患儿临床资料,包括发病时间、临床表现、诊疗方案和预后等。结果随访期间,393例儿童肾移植受者共8例(2.04%)发生PTLD。肾移植手术至PTLD发生的中位时间为4(3~14)个月,其中早发型7例、晚发型1例。7例以发热起病,5例以消化道症状起病。7例患儿术前EB病毒(EBV)血清学均阴性,确诊PTLD时8例均伴有EBV感染。6例病理提示单形性-B细胞型PTLD,1例为多形性PTLD,1例未行病理检查。早期诊断的2例患儿予利妥昔单抗(RTX)联合化疗,PTLD均获得缓解,其中1例患儿死于颅内真菌感染。后续6例患儿接受RTX及磺胺甲噁唑、泊沙康唑预防感染,PTLD均缓解,人肾存活。结论肾移植患儿随访中出现EBV感染、发热、消化道症状时需高度警惕PTLD。单形性-B细胞型及多形性PTLD予利妥昔单抗联合预防感染可取得满意治疗效果。
Objective To analyze the clinical characteristics and treatment of post-transplantation lymphoproliferative disorder(PTLD)in pediatric renal transplant recipients.Methods A retrospective analysis was conducted on the clinical data of children who developed PTLD during the follow-up after kidney transplantation at the Children's Hospital of Fudan University from January 2011 to June 2024,including the onset time,clinical manifestations,treatment plans,and prognosis.Results Eight recipients(2.04%)developed PTLD during follow-up,with a median onset interval of 4 months posttransplantation(range 3-14 months).Early-onset PTLD occurred in 7 patients,while 1 presented with late-onset PTLD.The most common initial manifestations were fever(n=7)and gastrointestinal symptoms(n=5).All 7 patients were serologically negative for epstein-barr virus(EBV)before surgery,and all 8 patients were associated with EBV infection when PTLD was diagnosed.Histopathological evaluation identified monomorphic B-cell PTLD in 6 patients and polymorphic PTLD in 1 case(1 patient undiagnosed due to missing biopsy).Initial management with rituximab(RTX)plus chemotherapy in two early-diagnosed patients achieved hematological remission;however,one patient succumbed to a fatal intracranial infection.Subsequent treatment protocol modification incorporating RTX with prophylactic antimicrobial therapy(sulfamethoxazole and posaconazole)in 6 patients resulted in complete PTLD remission with preserved graft function in all patients.Conclusions PTLD should be highly vigilant when EBV infection,fever and digestive tract symptoms occur in renal transplant children during follow-up.Monomorphic B-cell and polymorphic PTLD responded well to RTX combined with infection prevention,achieving satisfactory therapeutic outcomes.
作者
曾锦华
韩新利
徐虹
翟亦晖
刘锋
陈瑞
方晓燕
张致庆
王春燕
陈径
沈茜
Zeng Jinhua;Han Xinli;Xu Hong;Zhai Yihui;Liu Feng;Chen Rui;Fang Xiaoyan;Zhang Zhiqing;Wang Chunyan;Chen Jing;Shen Qian(Department of Nephrology and Kidney Transplantation,Children's Hospital of Fudan University,Shanghai Research Center of Kidney Development and Children's Kidney Disease,Shanghai 201102,China;National Key Laboratory of Kidney Diseases,Shanghai 201102,China)
出处
《中华移植杂志(电子版)》
2025年第1期22-25,共4页
Chinese Journal of Transplantation(Electronic Edition)
基金
东方英才计划拔尖项目(EK00000869)。
关键词
EB病毒
移植后淋巴组织增生性疾病
儿童肾移植
利妥昔单抗
Epstein-barr virus
Post-transplantation lymphoproliferative disorder
Pediatric kidney transplantation
Rituximab
